This patient commentary is written by Amanda Grandinetti, who works as a Senior Specialist, Performance Measures and Analysis for the American Academy of Dermatology. She is a transplant recipient with FSGS and the thoughts and recommendations are her own and not tied to her place of work.

I became very sick during the summer of 2004 while my family and I were vacationing in Virginia. I was 14 years old and about to start high school. I just had my mandatory high school physical with my primary care provider a few weeks prior, in which I learned I had proteinuria and was reassured that I was fine and that the proteinuria was normal for a 14-year old girl. Follow-up was not necessary.

Slowly over that summer, my family began to notice that my eyes seemed swollen in the morning. I began to gain weight as well, which was unusual for my 95 pound self. I woke up one morning during my vacation and my body had just exploded with edema everywhere. It was almost impossible to walk. My family brought me to a hospital, where I stayed overnight. The hospital told me I needed to follow-up with a nephrologist when I went home to Chicago.

I had no idea what was happening at the time. I did follow-up with a nephrologist back home. I was biopsied, put on Lasix, and remained on the prednisone. My biopsy results confirmed I had minimal change disease and I was told that I would one day outgrow this disease. Time passed and pounds of fluid were shed, but I could never get off the prednisone. My body became steroid dependent. A year after constant weening and failure, my team and I decided to try cyclophosphamide (Cytoxan). At this time, there were not many treatments dedicated to minimal change disease besides cyclophosphamide, tacrolimus, and prednisone. I lost almost all of my hair and completed the Cytoxan treatment but it did not work. My minimal change disease came back just five months after completing cyclophosphamide. I was in and out of the hospital for 6 months because I could not get into remission. I received another biopsy and this one showed that I had focal segmental glomerulosclerosis (FSGS). I began taking tacrolimus (Prograf) and miraculously reached remission, although it took about a year. I managed to live a relatively normal symptom free life on about 25-30 pills daily until I was 21. Then out of nowhere, the medication stopped working and I reached stage 4 kidney disease. I had not been taught the signs and symptoms of kidney failure and I had no idea that my kidney function was declining. No one was communicating what was happening to me and I was in a constant state of limbo.

After a miserable year in stage 4 CKD with acidosis, edema, ascites, and a hemoglobin of around six, I reached kidney failure. I had just turned 22. Eight years after I was told that I had 'normal' proteinuria, I was in kidney failure.

I was not educated about my risk of kidney failure when I was a teenager. In fact, I was terrified of it, and thought dialysis was just a step before imminent death. I did not learn what dialysis was until I had to start it. I was placed on a hemodialysis machine while screaming because I was terrified. I was not even educated on what it would feel like.

My patient experience shows that there is a gap in care on the education of pediatric patients that is specific to their life stage. I am a patient that is activated and involved in my care. I understand that not all patients are this activated. The responsibility of education falls onto providers and then falls into the hands of the patient.

My personal patient experience is relatable to Calderon-Margalit et al, 2018 because I had relatively normal kidney function until I was an adult. My experience is vastly different from the publication however, because I had severe symptoms which required monitoring throughout my childhood. Doctors never told me about my risk of kidney failure. It was a taboo subject. The lack of patient education left me unprepared for kidney failure. There may not be a way to fully prepare for kidney failure, but there is something to be said about the peace of mind from knowing what kidney failure entails, and knowing that I could live a full and happy life with kidney failure. Until that point, I assumed kidney failure meant I was going to die. I lived my life not thinking I would make it to my 20s. I am now 28 with a transplant and live a normal, healthy life with 100% kidney function.

I applaud Calderon-Margalit et al 2018. It is a very strong study that will hopefully bring light to the subject of the risk of kidney failure in adult patients with pediatric kidney problems. Possible misdiagnoses and the lack of specificity on specific glomerular diseases are the biggest limitations in the study. Missed diagnoses can be common in FSGS if the biopsy does not take a sample of tissue affected by scarring. As a next step, I would suggest a systematic review and meta-analysis of all available literature to make stronger recommendations, which can be incorporated into care delivery. This data may create a sense of urgency regarding education in pediatric kidney disease.

Questions and Recommendations:

Is primary care enough? I think a primary care provider is capable of monitoring an asymptomatic patient. I also think regular follow-up visits are enough to monitor care until it reaches a specific end point. What is this end point? We must come together and decide so we can standardize care. I have been involved in discussions regarding clinical end points for kidney disease and much of the discussion revolves around when patients should be referred out from primary care to nephrologists. Some focus on proteinuria, others focus on staging and various other clinical manifestations. It seems that stage 3 should be referred out to a nephrologist. Education on the detection of signs and symptoms is essential for primary care providers. Thorough follow-up instructions should be created for patients to guide them so that they may take action in the future and are equipped to deal with possible kidney problems.

Education can take many routes. When deciding the best route, it is important to ask yourself the following questions:

How was the illness discovered?

How long has it been present?

What were the first clinical signs of disease?- If the first clinical sign was swelling, a doctor could teach a patient to monitor for pitting edema in the legs.- If symptoms were absent and instead discovered through random testing, then the provider should discuss at length what signs and symptoms to look for.

What illness was the child diagnosed with?- If it is something that is typically harder to manage, as in the case of FSGS even when asymptomatic, it is probably best to refer out and be managed by a nephrologist.

How can we educate primary care providers?- Firstly, primary care providers need to do their due diligence when a problem arises. My provider did not follow-up on my proteinuria. It was immediately dismissed as normal. FSGS is associated with massive proteinuria. I am certain I had massive amounts of proteinuria when I received my physical exam for high school admission. We need to educate providers on what levels of proteinuria are acceptable and when to refer out. At what point may proteinuria be caused by normal activity, such as working out? I would believe that almost all proteinuria is a cause for concern and at least a follow-up should occur. Perhaps my kidney disease could have been managed easier with less steroids if it did not wait until I had massive amounts of edema. I believe the education of primary care providers (PCP) may fall onto the hands of nephrologists.

The nephrology world will also have to agree on when it is appropriate to transfer care from a PCP to a nephrologist. Education on prevention should be emphasized to PCP. PCP should be taught about medications that are nephrotoxic or metabolized in the kidney and offer patients alternatives to over-the-counter medications and prescriptions. Perhaps the patient with a history of kidney dysfunction but is symptom free can use acetaminophen instead of ibuprofen when a situation arises and use ibuprofen as a last case resort. Providers should be checking medications before prescribing them. Checking the disease history can inhibit additional silent damage to the kidneys. Even providers that knew I had kidney disease prescribed me medications that were harmful to my kidneys. Because I am such an activated patient, I made sure to tell the provider about my history and then check with the pharmacist and back with my nephrology team. It always alarmed me how many times this simple step was missed.

Having one universal electronic health record (EHR) is mentioned in the summary as a solution for finding accurate data. Unfortunately, we are very far away from interoperability in EHR. Until more regulations regarding transparency and solutions to end data blocking are created, significant barriers will remain. For now, interoperability remains just a hope for the future. Long patient records are also mentioned in the summary. I would argue that a provider should have to look through the medical records for a history of certain diseases. That is why medical records are kept. The review does not have to be completed by the actual provider. Perhaps a nurse or medical assistant can assist. I would also say this is a stronger argument for some practices to move from paper to EHR. There is a lot of debate regarding the administrative burden of having an EHR and the costs associated with them. I will defer this topic for now.

Recommendations: I would argue that standardization of management of pediatric patients should be the first step to finding data on how frequently providers are performing actions. There may be a sense that certain actions, such as checking medications, may be standard of care, but it does not seem that way. The data may reveal that it is not a standard of care and creating measures can help identify providers that need more education. Quality metrics and quality improvement programs can be created to get a sense of how much an action is performed. Measures can also create a new process for managing care. These metrics could exist in the pediatric or adult setting. There may be large concerns regarding loss to follow-up, but this is not an impossible task, as many performance measures have similar concerns. The same provider will most likely not follow the child into the adulthood. Perhaps we can create performance measures to identify patients with a history of kidney disease and then get an exam every year or two that includes education on the detection of signs and symptoms in this outpatient office visit. A simple process measure like this could identify patients who are at risk and providers who need to improve their process. Performance measures like this exist in many different houses in medicine, and are even used in the Merit-based Incentive Payment System (MIPS) through the Medicare Access and CHIP Reauthorization Act of 2015 (MACRA).